• James N George, Sara K Vesely, and Deirdra R Terrell.
• Department of Medicine, College of Medicine, The University of Oklahoma Health Sciences Center, Oklahoma City, 73190, USA.
• Semin. Hematol. 2004 Jan 1; 41 (1): 60-7.

AbstractThe Oklahoma Thrombotic Thrombocytopenic Purpura-Hemolytic Uremic Syndrome (TTP-HUS) Registry enrolls all consecutive patients for whom plasma exchange treatment is requested for clinically diagnosed TTP-HUS within a defined geographic region. During 14.5 years, from January 1, 1989 until June 30, 2003, 301 patients have been enrolled; follow-up is complete on 300 patients. Clinical categories have been designated based on associated conditions and potential etiologies; presenting features and clinical outcomes have been defined to allow comparisons between groups. ADAMTS-13 activity was measured on 142 (88%) of 161 consecutive patients enrolled from 1995 to 2001. Only 13% of all patients, and 33% of patients with idiopathic TTP-HUS, had severe ADAMTS-13 deficiency (<5% activity). The presenting features and clinical outcomes of patients with severe ADAMTS-13 deficiency were heterogeneous and not distinct from patients without severe ADAMTS-13 deficiency. These data suggest that severe ADAMTS-13 deficiency does not detect all patients who may be appropriately diagnosed with TTP-HUS and who may respond to plasma exchange treatment. Prospective data from consecutive patients are essential to translate new observations on pathogenesis into improved patient care.

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